Overview

Long QT syndrome (LQTS) is a heart rhythm condition that can potentially cause fast, chaotic heartbeats. These rapid heartbeats might trigger a sudden fainting spell or seizure. In some cases, the heart can beat erratically for so long that it causes sudden death.

You can have a genetic mutation that puts you at risk of being born with congenital long QT syndrome. In addition, certain medications, imbalances of the body's salts and minerals (electrolyte abnormalities), and medical conditions might cause acquired long QT syndrome.

Long QT syndrome is treatable. You might need to take medications to prevent an erratic heart rhythm. In some cases, treatment for long QT syndrome involves surgery or an implantable device.

You'll also need to avoid certain medications that could trigger your long QT syndrome. After treatment, you likely can live and thrive, even with this condition. You may be able to continue being active in recreational — and even competitive — sports.

Symptoms

Many people who have long QT syndrome don't have any signs or symptoms. You might be aware of your condition only because of:

Results of an electrocardiogram (ECG) done for an unrelated reason

A family history of long QT syndrome

Genetic testing results

For people who do experience signs and symptoms of long QT syndrome, the most common include:

Fainting. This is the most common sign of long QT syndrome. Long QT syndrome-triggered fainting spells (syncope) are caused by the heart temporarily beating in an erratic way. These fainting spells might happen when you're excited, angry, scared or during exercise.

You may lose consciousness without warning, for instance from being startled by a ringing telephone. If you have a normal fainting spell, you usually will have a warning sign first, such as lightheadedness, heart palpitations, irregular heartbeat, weakness or blurred vision. However, a fainting spell from long QT syndrome can occur with little to no warning.

Seizures. If the heart continues to beat erratically, the brain will eventually not get enough oxygen, which can cause seizures.

Sudden death. Generally, the heart returns to its normal rhythm. If this doesn't happen by itself, or if an external defibrillator isn't used in time to convert the rhythm back to normal, sudden death will occur.

Signs and symptoms of inherited or congenital long QT syndrome might start as a fetus, during the first weeks to months after birth, as late as older age, or never at all. Most people who experience signs or symptoms from long QT syndrome have their first episode by age 40.

Signs and symptoms of long QT syndrome might occur during sleep or arousal from sleep.

When to see a doctor

Consult your doctor if you suddenly faint during physical exertion or emotional excitement or after use of a new medication, especially if the medication is known to prolong the QT interval.

Because long QT syndrome can occur in families, also tell your doctor that you'd like to be tested for long QT syndrome if a first-degree relative (parent, sibling or child) has been diagnosed with long QT syndrome.

Causes

Chambers and valves of the heart

Chambers and valves of the heart

A normal heart has two upper and two lower chambers. The upper chambers — the right and left atria — receive incoming blood. The lower chambers — the more muscular right and left ventricles — pump blood out of your heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings.

Long QT syndrome is a heart rhythm disorder that can cause serious irregular heart rhythms (arrhythmias).

Normally your heart circulates blood throughout your body during each heartbeat. Your heart's chambers contract and relax to pump blood. These actions are controlled by electrical impulses that travel through your heart and cause it to beat. After each heartbeat, your heart's electrical system recharges itself in preparation for the next heartbeat.

In long QT syndrome, your heart muscle takes longer than normal to recharge between beats. This electrical disturbance, which often can be seen on an electrocardiogram (ECG), is called a prolonged QT interval.

Prolonged QT interval

Prolonged Q-T interval

Prolonged Q-T interval

A prolonged QT interval refers to an abnormality seen on an electrocardiogram. This abnormality reflects a disturbance in how your heart's bottom chambers (ventricles) conduct electricity.

An electrocardiogram (ECG) measures electrical impulses as they travel through your heart. Patches with wires attached to your skin measure these impulses, which are displayed on a monitor or printed on paper as waves of electrical activity.

An ECG measures electrical impulses as five distinct waves. Doctors label these five waves using the letters P, Q, R, S and T. The waves labeled Q through T show electrical activity in your heart's lower chambers (ventricles).

The space between the start of the Q wave and the end of the T wave (QT interval) corresponds to the time it takes for your heart to contract and then refill with blood before beginning the next contraction.

Doctors can measure whether the QT interval occurs in a normal amount of time. If it takes longer than normal to occur, it's called a prolonged QT interval. The upper limit of a normal QT interval takes into account age, sex, and regularity and speed of the heart rate.

Long QT syndrome results from abnormalities in the heart's electrical recharging system. However, the heart's structure is normal. Abnormalities in your heart's electrical system might be inherited. Or, they may be acquired due to an underlying medical condition or a medication.

Inherited long QT syndrome

At least 17 genes associated with long QT syndrome have been found so far, and hundreds of mutations within these genes have been identified. Mutations in three of these genes account for about 75 percent of long QT syndrome cases, while mutations in the other minor genes contribute a small percent of long QT syndrome cases.

About 20 percent of people who definitely have congenital long QT syndrome have a negative genetic test result. On the other hand, among families with genetically established long QT syndrome, between 10 percent and 37 percent of the relatives with a positive long QT syndrome genetic test have a normal QT interval.

Doctors have described two forms of inherited long QT syndrome:

Romano-Ward syndrome. This more common form occurs in people who inherit only a single genetic variant from one parent.

Jervell and Lange-Nielsen syndrome. This rare form usually occurs earlier and is more severe. In this syndrome, children inherit genetic variants from both parents. They have long QT syndrome and also are born deaf.

Additionally, scientists have been investigating a possible link between sudden infant death syndrome (SIDS) and long QT syndrome and have discovered that approximately five to 10 percent of babies affected by SIDS had a genetic defect or mutation for long QT syndrome.

Acquired long QT syndrome

Acquired long QT syndrome can be caused by certain medications, electrolyte abnormalities such as low body potassium (hypokalemia) or medical conditions. More than 100 medications — many of them common — can lengthen the QT interval in otherwise healthy people and cause a form of acquired long QT syndrome known as drug-induced long QT syndrome.

Medications that can lengthen the QT interval and upset heart rhythm include:

People who develop drug-induced long QT syndrome might also have some subtle genetic defects in their hearts. These defects make them more likely to have disruptions in their heart rhythm from certain medications.

Risk factors

People who may have a higher risk of inherited or acquired long QT syndrome may include:

Children, teenagers and young adults with unexplained fainting, unexplained near drownings or other accidents, unexplained seizures, or a history of cardiac arrest

Family members of children, teenagers and young adults with unexplained fainting, unexplained near drownings or other accidents, unexplained seizures, or a history of cardiac arrest

First-degree relatives of people with known long QT syndrome

People taking medications known to cause prolonged QT intervals

People with low potassium, magnesium or calcium blood levels — such as those with the eating disorder anorexia nervosa

Inherited long QT syndrome often goes undiagnosed or is misdiagnosed as a seizure disorder, such as epilepsy. However, long QT syndrome might be responsible for some otherwise unexplained deaths in children and young adults. For example, an unexplained drowning of a young person might be the first clue to inherited long QT syndrome in a family.

Complications

Most of the time, prolonged QT intervals in people with long QT syndrome never cause problems. However, physical or emotional stress might "trip up" a heart that is sensitive to prolonged QT intervals. This can cause the heart's rhythm to spin out of control, triggering life-threatening, irregular heart rhythms (arrhythmias) including:

Torsades de pointes — 'twisting of the points.' In this arrhythmia, your heart's two lower chambers (ventricles) beat fast and chaotically, making the waves on an ECG monitor look twisted. Less blood is pumped out from your heart, so less blood reaches your brain, causing you to faint suddenly and, often, without any warning.

If a torsades de pointes episode is short — lasting less than one minute — your heart can correct itself and you regain consciousness on your own. However, if a torsades de pointes episode lasts longer, it can result in a sudden fainting spell followed by a full-body seizure. If the dangerous rhythm does not correct itself, then a life-threatening arrhythmia called ventricular fibrillation follows.

Ventricular fibrillation. This condition causes the ventricles to beat so fast that your heart quivers and ceases pumping blood. Unless your heart is shocked back into a normal rhythm by a defibrillator, ventricular fibrillation can lead to brain damage and sudden death.

It's now known that long QT syndrome might explain some cases of sudden death in young people who otherwise appear healthy.

Prevention

If you have inherited long QT syndrome, be careful about which medications you take. Some medications — including certain appetite suppressants, decongestants and common antibiotics — might trigger dangerous heart rhythms. Ask your doctor what you can and can't take safely. Illegal drugs, such as cocaine and amphetamines, pose a serious risk for people with long QT syndrome.

In addition, seek medical treatment right away for illnesses that could result in low blood-potassium levels, especially if you have a lot of vomiting and diarrhea. Such illnesses could trigger an episode of long QT syndrome. Your doctor might advise you not to take some drugs, such as diuretics, that lower blood-potassium levels.

Some people — especially older adults with long QT syndrome who haven't had signs or symptoms of the condition in decades — may not need any treatment other than preventive measures.

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